Twin hearts-minimally invasive mitral valve repair in twin sisters with mitral annular disjunction and Loeys-Dietz syndrome: a case series.

In this case report, we present 31-year-old twin sisters diagnosed with severe Barlow mitral valve prolapse, mitral annular disjunction and presence of lateral mid-wall fibrosis diagnosed on MRI as well as ventricular arrhythmias, and a very rare variant of Loeys-Dietz syndrome, being referred to our center for surgical repair. Genetic testing detected pathogenic variants of clinical significance in SMAD3 and KCNH2 genes that are associated with autosomal dominant disease of Loeys-Dietz syndrome. Due to the presence of severe mitral valve regurgitation, the first patient was referred for minimally invasive mitral valve repair that was performed successfully. Before discharge, a subcutaneous ICD implantation was performed as primary prevention against malignant ventricular arrhythmias and sudden cardiac death. Her twin sister presented with the identical diagnosis and underwent the same surgical procedure with S-ICD implantation a few months later.

A Novel Approach for the Treatment of Pulmonary Artery Aneurysm Repair Using Inclusion Technique: A Case Report.

Pulmonary artery aneurysm (PAA) is a rare disease with life-threatening complications, especially when accompanied by pulmonary artery hypertension. Due to its rarity, there are currently no specific guidelines for the treatment of PAA. Several surgical techniques have been described to be beneficial in the treatment of PAA originating at the pulmonary trunk. However, several adverse complications have been described for traditional techniques. In this case, we present the first successful repair of PAA with idiopathic pulmonary artery hypertension using a graft inclusion technique.

Paediatric aortic valve replacement using decellularized allografts.

OBJECTIVES: Options for paediatric aortic valve replacement (AVR) are limited if valve repair is not feasible. Results of paediatric Ross procedures are inferior to adult Ross results, and mechanical AVR imposes constant anticoagulation with the inherent risks. METHODS: The study design was a prospective, multicentre follow-up of all paediatric patients receiving decellularized aortic homografts (DAHs) for AVR in 8 European centres. RESULTS: A total of 106 children (77 boys) were operated (mean age 10.1 ± 4.8 years, DAH diameter 20.5 ± 3.8 mm). A total of 60 (57%) had undergone previous surgical interventions: 34 with 1, 15 with 2 and 11 with ≥3. There was one early death in a 12-year-old girl, who underwent her fourth aortic valve operation, due to intracerebral haemorrhage on extracorporeal membrane oxygenation after coronary reimplantation problems following 3-sinus reconstruction 1 year earlier. One 2-year-old patient died due to sepsis 2 months postoperatively with no evidence for endocarditis. In addition, a single pacemaker implantation was necessary and a 2.5-year-old girl underwent successful HTx due to chronic myocardial failure despite an intact DAH. After a mean follow-up of 3.30 ± 2.45 years, primary efficacy end points mean peak gradient (18.1 ± 20.9 mmHg) and regurgitation (mean 0.61 ± 0.63, grade 0-3) were very good. Freedom from death/explantation/endocarditis/bleeding/stroke at 5 years was 97.8 ± 1.6/85.0 ± 7.4/100/100/100% respectively. Calculated expected adverse events were lower for DAH compared to cryopreserved homograft patients (mean age 8.9 years), lower than in Ross patients (9.4 years) and in the same range as mechanical AVR (12.8 years). CONCLUSIONS: Even though the overall number of paediatric DAH patients and the follow-up time span are still limited, our data suggest that DAHs may present a promising additional option for paediatric AVR.

Surgical Treatment of a Neuromucoepidermoid Carcinoma of the Left Main Bronchus via Posterolateral Thoracotomy in a 4-Year-Old Boy.

We report the case of a 4-year-old boy with a neuromucoepidermoid carcinoma of the left main bronchus. Complete resection of the carcinoma and reconstruction of the carina between lower and upper lobe by means of an end-to-end anastomosis was performed via a left-sided thoracotomy.

Twelve Years of Complete Atrioventricular Septal Defect Repair.

BACKGROUND: Surgical repair is the standard treatment for complete atrioventricular septal defect. At our institution, this repair is performed by single patch, modified single patch or two patch techniques, according to the surgeon preferences and the surgical anatomy of the defect. The goal of this study was to evaluate our results from the last twelve years. METHODS: From June 2006 to June 2018, 81 children with complete atrioventricular septal defect (without tetralogy of Fallot or unbalanced ventricles) were submitted to surgical repair at our institution. Data from all patients was retrospectively collected and evaluated. RESULTS: The average age was 6.9 ± 13.7 months and 84% had Down syndrome. Eighty percent were symptomatic and 6 patients were previously submitted to pulmonary artery banding. No more that mild left atrioventricular valve insufficiency was found in 84% and 89% of the patients, at discharge and follow-up, respectively. Small residual septal defects were present in 27% at discharge; during follow-up, 41% of these closed spontaneously. Pulmonary hypertension at discharge and follow-up appeared in 3.7% and 1.3%, respectively. Permanente pacemaker was implanted in 3 patients. Left ventricle outflow tract obstruction was found in 3 patients and 2 needed surgical correction. At follow-up (40 ± 38 months), 90% of the patients presented NYHA functional class I. No significant differences in the main repair outcomes were found between techniques, with the exception of small residual septal defects, although the groups were unmatched. CONCLUSIONS: Overall and regardless of the technique used for the repair of complete AVSD, good early and midterm outcomes were achieved.

The David procedure for surgical management of neo-aortic root dilatation following the arterial switch operation: Technical aspects.

Progressive neo-aortic/pulmonary root dilatation and associated valve regurgitation if translocated into the aortic position, remains a major drawback and serious late complication following the arterial switch operation. This video tutorial presents the technical aspects and a step-by-step illustration of the valve-sparing reimplantation technique, which  allows the neo-aortic valve to be maintained late after the arterial switch operation.

Pulmonary valve insufficiency 12 years after high-velocity blunt chest trauma.

The spectrum of cardiac injury due to blunt chest trauma may range from cardiac contusions with a clinically silent course to fatal cardiac wall rupture. Pulmonary valve insufficiency due to non-penetrating chest trauma is a rare entity. In this case, pulmonary valve insufficiency as a result of valve tear due to high-velocity blunt chest trauma required surgical replacement.

A Novel Designed Valved Conduit for RVOT Reconstruction in Grown-up Congenital Heart Patients: a Glimpse Down the Road.

BACKGROUND: A plethora of valves and valve conduits are available for reconstruction of the right ventricular outflow tract (RVOT) for grown-up congenital heart patients. However, for several reasons, the ideal pulmonary valve substitute still remains the subject of debate. In this study, we investigated the preliminary clinical and echocardiographic results after implantation of the RVOT Elan (Vascutek, Renfrewshire, United Kingdom) conduit in adolescents and adults. MATERIAL AND METHODS: Between October 2012 and December 2014, a total of 27 patients (19 males, mean age: 23.7 ± 22.5; range: 9-74 years) received a RVOT Elan conduit for RVOT reconstruction and were prospectively followed up clinically and echocardiographically. Twenty-five patients had previous cardiac surgery. The median number of prior operations per patient was 2 (range: 1-4). Tetralogy of Fallot was the most common diagnosis (n = 7). RESULTS: At a mean follow-up time of 0.9 ± 0.61 years (100% complete), all patients (27 of 27) were alive and in New York Heart Association Class I. Adverse events defined as valve failure, thrombosis, embolism, bleeding, or endocarditis did not occur. Freedom from reoperation in general was 100%. At 1-year follow-up, median peak pressure gradients (Δ Pmax) across the RVOT Elan conduit were 15 ± 3.2; 15.3 ± 2.1Δ, 16 ± 4.8, and 16.3 ± 5.1 mm Hg for the 19 (n = 3), 21 (n = 3), 23 (n = 6), and 25 mm (n = 15) conduit size, respectively. CONCLUSION: The RVOT Elan conduit revealed excellent preliminary clinical and hemodynamic performances independent from the underlying cardiac pathology with insignificant transvalvular gradients and nonturbulent flow characteristics.

Aortic valve/root procedures in patients with an anomalous left circumflex coronary artery and a bicuspid aortic valve: anatomical and technical implications.

An anomalous origin of the left circumflex coronary artery that arises as a side branch of the right coronary artery from the right coronary sinus of Valsalva encircling the aortic annulus is usually an incidental finding. However, in patients undergoing aortic valve procedures, its existence can significantly complicate the surgical treatment. We report our operative strategy in patients with an anomalous left circumflex coronary artery, a bicuspid aortic valve morphology and different aortic valve pathologies.

The David procedure for salvage of a failing autograft after the Ross operation.

BACKGROUND: Dilatation of the pulmonary autograft is a major fear after the Ross procedure. We analyzed the results after reoperation for autograft dysfunction using a valve-sparing reimplantation technique (David procedure). METHODS: From 1995 to 2012, 645 Ross operations were performed, with 630 (98%) of these as freestanding root replacements (mean follow-up, 8.3 ± 4.6 years). Forty-nine autograft reoperations occurred in 46 patients (0.89%/patient-year). Between 2005 and 2013, reoperation using a David procedure was performed in 18 of 35 patients (52%) with autograft dilatation at a mean interval of 11 ± 3.2 years after the Ross operation. RESULTS: The mean age of 18 patients receiving a David procedure as reoperation was 49.8 ± 13.9 years; 83% were male. The 30-day reoperative mortality was zero. The mean vascular graft size used for reimplantation was 29.5 ± 1.7 mm. At a mean follow-up time of 3.2 ± 2.3 years (100% complete), all patients (18 of 18) were alive and in New York Heart Association functional class I. One patient (5%) needed valve replacement for recurrent aortic regurgitation 2.6 years after the David procedure. In the remaining patients (95%), freedom from aortic regurgitation of grade 2 or greater was 100% at 3 years (regurgitation grade <1, 14 of 17; 82%). Aortic valve gradients were clinically insignificant at 5.8 ± 2.1 mm Hg. CONCLUSIONS: Performing a David procedure was successful in the vast majority of patients with dilatation of the pulmonary autograft after a Ross operation and revealed good function of the preserved autograft at midterm follow-up. Reoperations could be performed with low perioperative morbidity and mortality.

[Prevention and treatment of cardiac arrhythmia in patients with congenital heart defects: surgical aspects]. / Prävention und Behandlung von Herzrhythmusstörungen bei Patienten mit angeborenen Herzfehlern: Chirurgische Aspekte.

Catheter ablation procedures have become established over the years due to their effectiveness and low invasiveness and have changed the indications for surgical treatment. The main field of activity of cardiac surgery remains the treatment of patients with congenital heart defects which show arrhythmia and necessitate surgical treatment. The combination of surgical ablative measures with a good view of the site using modern ablation instruments, atrial reduction by tissue resection and correction of the defect with the resulting volume relief, bring the best conditions for a curative treatment of arrhythmia. The same applies to the treatment of ventricular tachycardia resulting from old scars and residual defects. Special attention should be given to patients who received a Fontan conversion. This operation is a unique opportunity to relieve volume which in turn reduces the atrial size and at the same time to take ablative measures to restore sinus rhythm. The results are promising with low mortality and morbidity, high efficiency and finally a clear improvement of the functional status of patients. In appropriate cases minimally invasive surgical procedures can help where pharmacological and catheter ablation therapies remain ineffective.

Quo vadis pulmonary autograft--the ross procedure in its second decade: a single-center experience in 645 patients.

BACKGROUND: The enthusiasm about the advantages of a viable autologous transplant faded with recent reports of autograft deterioration and associated reoperations after the Ross procedure. This report evaluates predictors for autograft failure and outcomes extending into the second decade after a Ross procedure. METHODS: From 1995 through 2012, 645 consecutive patients (mean age, 42.3 ± 14.2 years; 76% males) underwent a Ross operation using mainly the root replacement technique (98%). They were prospectively followed up with clinical and echocardiographic evaluations. Total follow-up was 5,349 patient-years and was 96% complete. Mean follow-up duration was 8.4 ± 4.6 years (range, 0 to 17.4 years). RESULTS: Early mortality was 0.9% (n = 6). Cumulative survival at 15 years was 92.7% (95% confidence interval, 90.1% to 95.3%) and did not differ from the general German population (p = 0.261). Freedom from reoperation on the autograft or the pulmonary allograft at 12 years was 91.6% (95% confidence interval, 88.5% to 94.9%) and 95.0% (95% confidence interval, 92.8% to 97.2%), respectively. Sixty-five patients (10.1%) required a total of 78 valve-related reoperations after a Ross procedure (1.5%/patient-year) with a reoperative hospital mortality of 3.8% (n = 3). Forty-seven autograft reoperations were observed in 44 patients (0.87%/patient-year); 22 of them (47%) could be performed as a valve-sparing procedure. Preoperative aortic valve regurgitation and an aortic annulus of at least 26 mm were identified as predictors for autograft failure. CONCLUSIONS: In this large series, the Ross procedure resulted in excellent long-term survival rates with a low risk of valve-related morbidity and a considerably low rate of reoperations in young and middle-aged patients and should be considered as an important treatment option in this cohort.